title: Longitudinal analysis of heart and liver iron in thalassemia major patients according to chelation treatment.
abstract: Iron chelators and nuclear magnetic resonance imaging (MRI) techniques for assessing iron loading in liver and heart have greatly improved survival of thalassemic patients suffering iron overload-associated cardiomyopathy. However, the correlation between liver iron concentration and myocardial siderosis is ambiguous. Using an objective metric of time delay, scientists have demonstrated a lag in the loading and unloading of cardiac iron with respect to that of the liver. In the present study, we further tested this hypothesis with different chelation treatments. We analyzed the effect of three chelating treatment approaches on liver and cardiac iron content in 24 highly compliant patients who underwent 3 or more MRIs under each chelation treatment. Of the 84 MRIs considered, 32 were performed on deferoxamine (DFO - 8 patients), 24 on deferiprone (DFP - 7 patients), and 28 on combined therapy (DFO+DFP - 9 patients). In patients treated with DFO, changes in cardiac iron significantly lagged changes in liver iron but the opposite pattern was observed in patients treated with DFP (p=0.005), while combined therapy showed a pattern in-between DFO and DFP. We conclude that the temporality of changes of cardiac and liver iron is chelator-dependent, so that chelation therapy can be tailored to balance iron elimination from the liver and the heart.
authors.affiliation: Dipartimento di Sanità Pubblica, Medicina Clinica e Molecolare, Sezione di Scienze Biomediche e Biotecnologie, Università di Cagliari, Ospedale Regionale per le Microcitemie ASL8, Cagliari, Italy. fabdanjou@tiscali.it
authors.forename: F
authors.initials: F
authors.lastname: Danjou
authors.forename: R
authors.initials: R
authors.lastname: Origa
authors.forename: F
authors.initials: F
authors.lastname: Anni
authors.forename: L
authors.initials: L
authors.lastname: Saba
authors.forename: S
authors.initials: S
authors.lastname: Cossa
authors.forename: G
authors.initials: G
authors.lastname: Podda
authors.forename: R
authors.initials: R
authors.lastname: Galanello
mesh_terms: D000328:Adult; D015913:Chelation Therapy; D006801:Humans; D007501:Iron / Q000378:metabolism*; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D008099:Liver / Q000378:metabolism* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D009206:Myocardium / Q000378:metabolism* / Q000473:pathology; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications*
chemical_list: D007502:Iron Chelating Agents; D007501:Iron
keywords: Beta-thalassemia; Heart siderosis; Iron chelation; Liver iron concentration; Magnetic resonance